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Abstract

Traditionally Motor Neurone Disease (MND) has been thought of as a syndrome
exclusively affecting the motor regions of the cortex only. However, recent research
has found evidence of extra-motor involvement in patients with the classical form of
MND predominately on tests of executive dysfunction. This selective cognitive
dysfunction found in classical MND patients, namely on tests of executive function
and to a lesser extent on memory and language functions, has led to the hypothesis
that MND is a spectrum disorder. It follows, that if ALS and MND-D are part of a
continuum then evidence of subtle behavioural change characteristic of MND-D and
FTD consistent with orbitofrontal dysfunction should be observed in the nondemented
ALS patient group. The research aims of this study were therefore to
investigate whether patients with non-demented ALS do in fact display subtle
behavioural change associated with orbitofrontal dysfunction characteristic of
frontotemporal dementia. A neuropsychological battery including tests of behavioural
dysfunction was administered to 10 ALS patients and 10 healthy controls. The cares
of each patient were also asked to complete three informant-based questionnaires in
order to assess patients’ behavioural profile. Evidence was presented of subtle
behavioural change in this patient group in the domains of apathy, disinhibition and
executive dysfunction as well as evidence of behavioural features associated with the
FTD diagnostic criteria set out by Neary and colleagues (1998). This evidence adds
further weight to the hypothesis that ALS, MND-D and FTD form variants of a
clinical pathological continuum. Further investigations are necessary to confirm
theses findings, with the possible use of imaging techniques to provide evidence of
cerebral abnormality in a similar patient group.